节段性狭窄闭塞布-加综合征的介入治疗

目的　探讨布 加综合征 (BCS)下腔静脉节段性狭窄、闭塞合并肝静脉阻塞的介入治疗。方法　 2 8例节段性狭窄、节段闭塞患者 ,多数合并 1～ 3支肝静脉闭塞 ,无合并下腔静脉血栓形成。应用球囊导管或房间隔穿刺针行下腔静脉开通术 ,下腔静脉开通后用 2 .0～ 2 .3cm球囊扩张后放入金属内支架。术后抗凝治疗 2年。结果　 2 7例患者成功进行了经皮穿刺球囊破膜扩张 ,即经皮腔内血管成形术 (PTA)和血管内支架植入治疗。闭塞肝静脉多数扩张下腔静脉后同时开通。患者肝、脾缩小 ,腹水吸收。 1例穿破下腔静脉急诊手术止血。随访 2～ 36个月未见复发及消化道出血。结论　下腔静脉狭窄者注意有肝静脉闭塞 ;下腔静脉闭塞破膜后 ,2 0例肝静脉闭塞者 19例随之开放 ,无需特殊处理 ;安放支架时注意保护肝静脉入口。     

Spontaneous CSF leaks mimicking benign exertional headaches

Spontaneous CSF leaks are increasingly recognized, and a broader clinical and imaging spectrum of the disorder is emerging. The headaches of CSF leaks are typically orthostatic, but sometimes especially with chronicity the orthostatic features are blurred into lingering chronic daily headaches. Additional types of headache are also increasingly recognized. Two patients with spontaneous CSF leaks presented with intermittent transient severe headaches provoked by Valsalva-type manoeuvres. Orthostatic features were absent and the patients were asymptomatic if they avoided the provoking manoeuvres. One patient had been treated for 6 years for benign exertional headaches and had failed many medical treatments, including courses of indomethacin. He was found to have a leak from cribriform plate. The second patient had been symptomatic for several months, had diffuse pachymeningeal gadolinium enhancement on head magnetic resonance imaging, spinal meningeal diverticula, and CSF leak at the thoracic spine level. Headaches that mimic benign exertional headaches are yet another mode of the still broadening clinical presentation of spontaneous CSF leaks.     

Unusual hepatic-portal-systemic shunting demonstrated by Doppler sonography in children with congenital hepatic vein ostial occlusion

PURPOSE: This report describes unusual changes in the hepatic vasculature in 3 children presenting with upper gastrointestinal hemorrhage. METHODS: The study included 3 children (ages 5-8 years) who presented with hematemesis. All had mild hepatosplenomegaly and normal liver function. Esophageal varices were demonstrated in all on upper endoscopy. Color and spectral Doppler sonography was performed to assess the hepatic vasculature, including the hepatic veins (HVs), portal vein (PV), hepatic artery (HA), and inferior vena cava (IVC). RESULTS: The HVs were all patent but with ostial occlusion at the point of their communication with the IVC. Complete flow reversal was shown inside the HVs, with blood draining into collateral vessels at the liver surface and paraumbilical vein. In one patient, the paraumbilical vein could be traced to its communication with the right external iliac vein. In all children, the direction of flow in the PV, HA, and IVC was normal. After endoscopic sclerotherapy, all children were shown to be in good general condition and to have normal liver function for a follow-up period of 15-36 months. CONCLUSIONS: Ostial occlusion of the HV is a rare cause of hepatic outflow obstruction in children. Doppler sonography is a valuable, noninvasive imaging technique for evaluation of the hepatic vasculature and the accompanying shunting pathways in such cases.     

Arnold-Chiari畸形Ⅰ型的MR诊断价值

目的：探讨MR对Arnold-Chiari畸形Ⅰ型的诊断价值。材料与方法：19例ACMⅠ型均经MPd诊断，手术及临床证实，其中：男8例，女11例，年龄18—59岁，平均36岁。结果：12例小脑扁桃体疝入枕大孔平面下5mm-28mm，5例2mm-4mm，11例颅颈部各种畸形，18例扁桃体下极变尖与扁桃体前池变形，11例颈扁桃体痈池消失和／或延髓扭曲成角，12例伴有脊髓空洞，6例脑积水，1例后颅凹脑膜瘤。结论：MR成像技术是Arnold-ChiariⅠ型畸形诊断的首选检查诊断方法；Arnold-ChiariⅠ型畸形的早期诊断对患者预后至关重要。颅颈部MPI测量对Arnold-ChiariⅠ型畸形的诊断、治疗及预后有重要价值。     

Entrapment of a Pacing Lead within a Chiari Network: Utility of Intracardiac Echo and a Laser Sheath

Although rare, Chiari networks are elaborate embryological remnants that can pose distinct challenges for catheter and pacing lead manipulation within the right atrium. Device entrapment may require open thoracotomy for removal, with significant morbidity. We report an unusual case of pacing lead entanglement within this structure, followed by prompt intracardiac echocardiographic identification and laser sheath removal.     

Suboccipital craniotomy in the surgical treatment of Chiari I malformation

Object  The object of this study was to present craniotomy for Chiari type I patients. Materials and methods  Six patients with Chiari type I underwent suboccipital craniotomy. All patients showed clinical improvement, and none had any complications. Two patients had syringomyelia; it disappeared in entirety. We describe the procedure for posterior fossa decompression. Results  Three-dimensional volumetric analysis using Vitrea workstation for postoperative posterior fossa volumes was calculated and was seen to have been increased on an average, from pre-operative (168 cc) to postoperative volume (192 cc). Conclusion  We thus conclude that suboccipital craniotomy results in resolution of the Chiari symptoms yet achieves effective expansion of posterior fossa.     

Cervical spine instability following cervical laminectomies for Chiari II malformation: a retrospective cohort study

Objective  The treatment of symptomatic Chiari II malformations typically involves multilevel cervical laminectomies in very young children. These patients are at significant risk of cervical instability. The purpose of this study was to determine the incidence and significance of cervical instability after multilevel cervical laminectomies in a cohort of patients decompressed for Chiari II malformation. Methods  Postoperative dynamic lateral cervical spine radiographs were obtained on pediatric patients who had multilevel cervical laminectomies for symptomatic Chiari II malformations. Postoperative cervical spine instability was determined radiographically using published criteria. Clinical instability and need for cervical fusion were also assessed. Results  Nine patients met inclusion criteria for the study. Five of the nine patients (56%) showed evidence of radiographic instability of their cervical spines following surgery for their Chiari II malformations, according to the criteria used. No patient showed evidence of clinical instability or required cervical fusion. Conclusion  Radiographic evidence of cervical spine instability following multilevel cervical laminectomies for Chiari II is common but may be of minimal clinical significance. The reason for the lack of clinical instability in what might be considered high-risk patients is not understood.     

Hepatic vena cava disease: Etiologic relation to bacterial infection

Aim: Hepatic vena cava disease is a primary obliterative disease of the hepatic portion of the inferior vena cava (IVC) that often causes liver cirrhosis and hepatocellular carcinoma. Its geographic prevalence is inversely related to the standard of community hygiene. The disease is endemic in Nepal and is commonly associated with bacterial infection. The cause of the disease is not known. It was previously thought to be congenital. Thrombosis due to hypercoagulable condition is suggested as a possible cause of the disease. This study looks at the relation of the disease to bacterial infection. Methods: Ultrasonography is sensitive and specific for the diagnosis of acute and chronic lesions of hepatic vena cava disease. Patients attending the Liver Clinic with pyrexia with and without bacteremia were examined by ultrasonography for acute "thrombophlebitic" lesion of the IVC, and five patients with bacterial infection with acute lesion in the hepatic portion of the IVC were followed. Results: Sixty eight percent of the patients with bacteremia had ultrasonographic evidence of acute lesion in the hepatic portion of the IVC, compared to 18% patients without bacteremia. A follow-up study of five patients showed transformation of the acute lesion into chronic obliterative lesion - stenosis or complete obstruction. Conclusion: Bacterial infection is probably the cause of hepatic vena cava disease seen in developing countries.     

两种手术方式对Chiari畸形Ⅰ型合并脊髓空洞的系统评价及meta分析

目的 系统评价后颅窝减压（posterior fossa decompression,PFD）和后颅窝减压加硬膜成形术（posterior fossa decompression plus duraplasty,PFDD）在Chiari畸形Ⅰ型合并脊髓空洞患者中的有效性及安全性。方法 检索Embase、Cochrane、Pubmed、Ovid、Medline、ScienceDirect、谷歌学术、万方、知网等数据库自建库到2019年的文献,筛选文中对PFD与PFDD进行了比较的随机对照研究或非随机对照研究,统计术后临床症状和影像学改善情况、并发症等指标,运用Revman（5.3版本）进行数据分析。结果 最终纳入10篇文献,共3 188例,PFDD组1 383例,PFD组1 805例。在脊髓空洞改善率（OR：5.53;95%CI：2.86,10.69）、症状缓解率（OR：2.53;95%CI：1.30,4.91）、并发症发生率（OR：3.46;95%CI：1.40,8.59）、脑脊液漏发生率（OR：9.36;95%CI：2.63,33.34）、假性硬脑膜膨出率（OR：1.89;95%CI：1.28,2.79）方面PFDD组高于PFD组（P<0.05）。在切口感染发生率（OR：1.44;95%CI：0.57,3.59）、皮下积液发生率（OR：1.71;95%CI：0.50,5.80）方面两种术式无差异（P>0.05）。结论 针对Chiari畸形Ⅰ型合并脊髓空洞的患者,PFDD的有效性优于PFD。     

Chronic migraine and chronic tension-type headache: are they the same or different?

Posterior cranial fossa tumours, not involving the cerebellopontine angle cistern, are a rare cause of trigeminal neuralgia (TN). We describe a patient with a large paramedian tentorial meningioma associated with acquired Chiari malformation who presented with TN. Trigeminal pain resolved after gross total tumour resection and postoperative magnetic resonance images disclosed a minimal residual tumour in the torcular region as well as ascent of cerebellar tonsils. In this article, we investigate the physiopathological hypotheses for this unusual association with emphasis on the role of tonsillar prolapse as neuropathological basis of neuropathic pain in this patient.